AB1074 FATAL BUT PREVENTABLE - SINGLE CENTRE SERIES OF 6 CASES OF PNEUMOCYSTIS JIROVECII PNEUMONIA (PJP) IN PATIENTS WITH AUTO-IMMUNE INFLAMMATORY RHEUMATIC DISEASE (AIIRD) IN ONE YEAR.
Authors: A. Escudero Siosi, M. Andersson, C. Woodrow, S. Dubey
Journal: Annals of the Rheumatic Diseases (2022)

Background Pneumocystis jirovecii pneumonia (PJP) is an uncommon but frequently fatal fungal infection, which can affect patients with rheumatic diseases treated with immunosuppressants or high doses of steroids. There are no clear guidelines on when to prescribe primary prophylaxis and available agreements differ depending on the disease or immunosuppressant. Objectives To raise awareness about this preventable infection and to highlight the urgent need to create a tailored probability scoring, before starting any immunosuppression so that the risk benefit of prophylaxis can be objectively assessed. Methods This is a retrospective case series of six patients who developed definite or probable PJP known to the Rheumatology Department at Oxford University Hospitals NHS FT since the beginning of 2021. These patients were identified through the microbiology and infectious disease teams, and notes were reviewed to collate data regarding the clinical characteristics. Of these, five were being treated for large vessels vasculitis (LVV) whilst the other one had seropositive rheumatoid arthritis (RA). The diagnosis of PJP was made on clinical picture, laboratory results, bronchoscopy and CT findings. Results In this series, the median age was 78 years (range 55-93) with equal gender distribution. In three LVV patients, the diagnosis was confirmed on ultrasound, one had a positive PET-CT whilst the other case had a high probability clinical diagnosis. Comorbidities included chronic kidney disease and hypertension in three patients, diabetes, or previous underlying malignancy in other two. Smoking history was present in four patients, while five patients had lymphopenia with counts &lt;1x10^9/L. Four of the six cases were on combined therapy with disease modifying anti-rheumatic therapy drugs (DMARDs) and prednisolone, only one was exclusively on prednisolone and the patient with RA was on Methotrexate and Humira. The chronology of the infection was variable, still most of the patients developed PJP infection during the first three months of starting either a biologic or corticosteroids. The median steroids dose by the time of PJP infection was 30 mg and unfortunately three of the patients died. None of the patients who developed PJP had been given antibiotic prophylaxis prior to infection. Some proposed scoring systems for serious infection risk in patients with AIIRD exist, however they focused on RA or biologics use rather than patients with vasculitis or connective tissue disorders who might be on high dose corticosteroids. Additionally, PJP prophylaxis is not recommended in any of the current guidelines for LVV management (BSR, EULAR, ACR). Cochrane review suggests reduction of risk by 85% in patients given prophylaxis. Conclusion Pneumocystis Jirovecii pneumonia (PJP) prophylaxis is not current practice for patients with large vessel vasculitis. Consideration needs to be given to PJP prophylaxis for patients on high dose steroids for a prolonged period, particularly in the presence of other risk factors. More data will be needed to help establish guidelines on PJP primary prophylaxis. References [1]Park JW, Curtis JR, Moon J, Song YW, et al. Prophylactic effect of trimethoprim-sulfamethoxazole for pneumocystis pneumonia in patients with rheumatic diseases exposed to prolonged high-dose glucocorticoids. Ann Rheum Dis. 2018 May;77(5):644-649. [2]Stern A, Green H, Paul M, Vidal L, Leibovici L. Prophylaxis for Pneumocystis pneumonia (PCP) in non-HIV immunocompromised patients. Cochrane Database Syst Rev. 2014 Oct 1;2014(10) Table 1. Baseline characteristics of the cases (*n) Male gender 3 Age, year Median 78 Underlying disease Large vessels vasculitis 5 Confirmed on imaging (n) 4 Seropositive Rheumatoid arthritis 1 Smoking 4 Lymphopenia 5 Steroid dose ≥ 30 mg by the time of PJP infection 4 Concomitant DMARDs used 4 Numbers of deaths 3 *n = numbers Disclosure of Interests None declared